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Cushing’s syndrome

Cushing’s syndrome. Elżbieta Petriczko. Physiology of adrenal cortex:. Adrenal cortex produces: 1) glucocorticoids (eg cortisol ) which affect metabolic functions ( carbohydrate, lipid and protein metabolism) mineralocorticoids (eg aldosterone ) androgens and oestrogens. ACTH.

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Cushing’s syndrome

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  1. Cushing’s syndrome Elżbieta Petriczko

  2. Physiology of adrenal cortex: • Adrenal cortex produces: • 1) glucocorticoids (eg cortisol) which affect metabolic functions ( carbohydrate, lipid and protein metabolism) • mineralocorticoids (eg aldosterone) • androgens and oestrogens

  3. ACTH Cushing syndrome ACTH dependent independent 12% 10% 8% 70% ACTH ACTH ACTH adenoma carcinoma Cortisol

  4. Clinical features: • 1) skin changes: • acne • seborrhea • increased growth of hair, especially pubic hair • appearance of dark-colored striae due to weakening of collagenous connective tissue in the skin • wasting of tissues ( myopathy, thin skin) • truncal obesity ( waight gain 90%) due to increased protein catabolism and loss of muscle mass • the buffalo-hump is characteristic ; the latter is due to increased fat padding between the scapulae • vascular changes consist of • hypertension ( 50% to 80%) • increased capillary fragility • and easy bruising • abnormality of bone matabolism • with demineralization of the skeleton lead to pathological fractures • osteoporosis, • growth retardation is common

  5. Clinical features: • 7) water retention, oedema, • plethoric moon-face, (broadening of the face with • temporal fat padding, chubby cheeks and double chin) • 9) predisposition to infection, ( bacteria , fungi, parasites) • 10) poor wound healing, • 11) hirsuitism, • 12) amenorrhoea, • 13) hyperglicaemia (30%) ; rarely , children may present with • polyuria, polydipsia (resulting from glucocorticoid excess • causing hyperglicaemia) • 14) psychological disturbances ranging from mild personality • changes to severe psychosis • 15) growth retardation ( 83%) and delayed puberty • 16) hyperpigmentation may be present with ACTH excess • 17) virilization ( if the adrenal tumor is producing androgens • as well as glucocorticoids) : premature pubarche, • clitoromegaly, penile enlargement

  6. Clinical features in children: • Cushing’s syndrome is due to chronic glucocorticoid excess. • The condition is rare in childhood. • In infants and children one may not find the marked difference in obesity between the trunk and extremities that is often noted in adults. • Short stature may be the only presenting symptom in some children. • When Cushing’s syndrome occurs before 7 years, it typically implies the presence of an adrenal tumor. • In contrast, after 7 years, most cases are due to increased ACTH-secreting pituitary tumor may be found.

  7. Screening tests (out patient): • 24h urine collection for urine free cortisol assay. • Normal <700 nmol/24h or 25 to 75 ug/m2/24hr. • In Cushing’s this is raised • More than one collection is needed. !!!! * 3% of normal individuals have levels of urinary free cortisol exceeding the normal range on a single determination. * Normal values have been reported ion patients with confirmed Cusing syndrome.

  8. Inpatient tests: • Plasma cortisol measured at 22.00 h and 09.00 h • The normal plasma cortisol concentration is • 5-25 ug/dl ( 140 to 690 nmol/L in early morning, dropping to less than 50% in the late evening ( 11.00 PM to 12.00 midnight) • Loss of diurnal variation is typical of Cushing syndrome. • The false-negative rate of this test is only 3%. • This pattern of diurnal variation often is not yet established in children less than 3 years old. • Cusing’s suggested if level at 22.00h is about equal to that at 09.00h ( it is normally lower).

  9. LABORATORY EVALUATION: • fasting hypergyceamia • normal sodium level • normal or low potassium level (resulting from the mineralocorticoid effect of high cortisol levels) • hypercalciuria (40%) with normal serum calcium and phosphorus • hyperinsulinemia • elevation of plasma lipoproteins • leukocytosis and lymphopenia

  10. HORMONAL TESTS: • No test has 100% sensitivity or specificity, • …… so multiple testing in the same patient is necessary to confirm a specific diagnosis.

  11. Short dexametazone test Dexametazon 1 mg cortisol (plasma) < 5 mg/dl 2200 800 • (-) • test only confirm the hypercortisolis • 5% false (-) • 15 – 20% false (+)

  12. ACTH Cushing disease ♀:♂ 8:1 80-90 % microadenoma (<10 mm). ~50% < 5 mm ~1% adenoma out of pituitary gland ACTH Cortisol

  13. microadenoma Normal MRI after Gd-DTPA

  14. Cushing disease A.K. ♀ l. 55 MRI:pituitary gland small, without signs of microadenoma presence diagnosis: microadenoma near pituitary gland

  15. ACTH ACTH ectopic ACTH production: ♀:♂ 1:5 rare 50% bronchogenic carcinoma (~2% cases of ACTH- dependent Cushing syndrome) pancreas carcinoid pulmonis, thymi, pancreas, oviaries pheochromocytoma CRH Cortisol

  16. Reasons of the elevated ACTH value ACTH pg/ml 3000 1000 500 100 60 20 NORMA Cushing disease Adrenal insufficiency Cushing syndrome CAH Nelson syndrome ectopic ACTH production

  17. Cushing disease A.T. ♂ 40 l. TBC death

  18. Cushing disease R.S. ♂ lat 30 since10 years hypertension stroke Epilepsia and left hemiparesis

  19. Cushing disease A.K. ♂ lat 30 serious osteoporosis. 1 year height 170  160 cm

  20. Treatment: • In the case of excessive pituitary ACTH release, management includes transsphenoidal microsurgery, with a remission rate of 85%-95%. • Irradiation of the pituitary gland –successful in 80%

  21. The risk of hypopituitarism is high after irradiation. • During and after surgery, glucocorticoid replacement is essential. • TREATMENT of adrenal gland tumors consist of surgical removal with replacement of glucocorticoids until the remaining adrenal gland is functioning normally.

  22. Pharmacotherapy Ketokonazol 600 – 1200 mg daily inhibit cytochrome P-450 action and cholesterol production Metyrapon inhibit 11b-hydroksylase Aminoglutetymid inhibit cholesterol conversion Mitotan

  23. Cushing disease L.D. ♀ l. 39 Two years of diagnostic procedures: suspected dermatmiositis Before treatment After pituitary adenoma removal

  24. Cushing disease B.D. ♀ l. 39 Four years of treatment because of: hirsutismus secondary amenorrhea Before treatment After pituitary adenoma removal

  25. Cushing diasease M.S. ♀ l. 46 Two years of depression treatment: Before treatment After pituitary adenoma removal

  26. ACTH-dependent Cushing syndrome A.B. ♂ lat 21 After pituitary operation After bronchocarcinoma removal

  27. ACTH-zależny zespół Cushinga J.P. ♂ lat 60 RTG: zmiany pogruźlicze TK:guzy obu nadnerczy meta meta rak drobnokomórkowy ACTH: 790 pg/ml CgA: 168 U/l

  28. ACTH-zależny zespół Cushinga A.B. ♂ lat 21 oktreoscan TK kl. piersiowej Guz (rakowiak) prawego płuca skutecznie przygotowany analogami somatostatyny do operacji Po operacji mikrogruczolaka przysadki

  29. ACTH-zależny zespół Cushinga B.D. ♂ lat 47 Guz (rakowiak) lewego płuca skutecznie przygotowany analogami somatostatyny do operacji Hydrocortison 30 mg/d ACTH pg/ml kortyzol mg% Sandostatin LAR 30 mg co 3 tyg. 200 80 O P E R A C J A 60 100 40 20 10

  30. ACTH-zależny zespół Cushinga Z.R. ♂ lat 41 Po operacji przysadki TK kl. piersiowej oktreoscan

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